China
Africa
Explore chapters and articles related to this topic
Retinal Vein Thrombosis
Published in K. Gupta, P. Carmichael, A. Zumla, 100 Short Cases for the MRCP, 2020
K. Gupta, P. Carmichael, A. Zumla
There are several conditions which predispose to retinal vein thrombosis: Hyperviscosity syndrome (myeloproliferative disorders, myeloma).Hypertension.Diabetes mellitus.Glaucoma and hypermetropia.
Waldenström Macroglobulinemia
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Anemia, seen in more than 30% of the patients, is multifactorial and secondary to bone marrow infiltration by LPL leading to inadequate erythropoiesis, IgM-associated hemolysis, and decreased levels of erythropoietin [57]. The production of erythropoietin is inversely related to plasma viscosity, which can be increased in WM patients with high levels of IgM paraprotein [58]. Overproduction of hepcidin has also been described as a contributing factor for anemia in patients with WM [59]. Hyperviscosity syndrome (HVS) has also been reported in approximately one-third of the patients. IgM immunoglobulins form large pentamers, restricted mostly to the intravascular compartment, leading to increased blood viscosity when IgM immunoglobulins are present in large quantity. The level of IgM that triggers HVS is variable among patients, as the final blood viscosity is influenced by multiple factors, such as hydration status and hemoglobin/RBC mass. The usual manifestations of HVS are mucocutaneous bleeding, visual disturbances, dizziness, headache, ataxia, tinnitus, exacerbation of heart failure, and rarely stroke or altered mental status [57,60]. Visual disturbances in HVS are usually secondary to retinal hemorrhages, papilledema, and central retinal vein thrombosis [61].
Lymphocyte and plasma cell malignancies
Published in Gabriel Virella, Medical Immunology, 2019
Juan Carlos Varela, Gabriel Virella
Some plasma cell dyscrasias may present with a constellation of symptoms known as the hyperviscosity syndrome. This is a direct result of increased serum viscosity, caused by high concentrations of monoclonal proteins. Serum viscosity is directly related to protein concentration. IgM and polymeric IgA, due to their molecular complexity and high intrinsic viscosity, lead to disproportionate increases of blood viscosity (Figure 27.4). Not surprisingly, the hyperviscosity syndrome is a frequent manifestation of Waldenström's macroglobulinemia, a B-cell dyscrasia defined by the synthesis of monoclonal IgM. However, the hyperviscosity syndrome is also observed in multiple myeloma patients, mainly in those with polymeric IgA paraproteins, and occasionally in IgG myeloma, when the concentrations of IgG are very high. The symptoms of serum hyperviscosity are related to high protein concentration, expanded plasma volume, and sluggishness of circulation. Table 27.1 lists the main signs and symptoms of the syndrome. Typical funduscopic changes are shown in Figure 27.5.
The Spectrum of Ocular Manifestations in Patients with Waldenström’s Macroglobulinemia
Published in Ocular Immunology and Inflammation, 2022
Rosanna Dammacco, Walter Lisch, Tero T. Kivelä, Evangelos Terpos, Efstathios Kastritis, Dario Sisto, Alberto Mavilio, Roberto Ria, Giovanni Alessio, Angelo Vacca, Franco Dammacco
Among the clinical manifestations of WM, hyperviscosity syndrome (HVS) merits particular attention. Given the pentameric structure and high molecular weight (970 kDa) of IgM molecules, their increased serum concentration in WM, and their interaction with red blood cells (responsible for rouleaux formation), hyperviscosity may become symptomatic either at the time of diagnosis or at any point during the disease in 10–36% of patients.2,10–12 In addition to the already mentioned constitutional symptoms and neurologic disturbances, such as headache, vertigo, dizziness, somnolence, and impaired mentation, hyperviscosity-related manifestations include epistaxis, gingival and mucocutaneous bleeding, gastrointestinal hemorrhages, congestive heart failure, renal impairment, visual disturbances, and other vascular abnormalities.2,12,13
Immunoglobulin G kappa multiple myeloma with the only initial manifestation of Raynaud’s phenomenon
Published in Scandinavian Journal of Rheumatology, 2020
C-C Chu, Y-H Chiu, M-H Lin, J-H Yeh, H-C Chen, S-Y Kuo
RP is not a typical presentation for malignant disease; it is termed ‘paraneoplastic RP’ in this condition . Although rare, carcinoma of lung, ovary, testis, thyroid, skin, and haematological malignancy can cause paraneoplastic RP. The precise prevalence of RP in patients with multiple myeloma remains unclear. Hyperviscosity syndrome is found in about 4% of patients with multiple myeloma (2). Hyperviscosity syndrome usually presents in IgM myeloma caused by large molecular weight immunoglobulins and excessive paraprotein concentrations. Increased viscosity in a low molecular weight multiple myeloma, such as an IgG or IgA myeloma, is even rarer. Aggregation with polymerization of the IgG may have contributed to high serum viscosity in this patient (3). Most commonly, hyperviscosity syndrome leads to neurological symptoms, heart failure, or bleeding tendency. RP in this patient was considered to have occurred owing to hyperviscosity. Gammopathy-related hyperviscosity explained the poor treatment response to our initial vasodilator therapy. Plasmapheresis is the most effective way to relieve symptoms in patients with refractory hyperviscosity syndrome (3). Our patient was free from RP after bortezomib therapy; therefore, plasmapheresis was not performed.
Diagnosis of Waldenström macroglobulinemia
Published in Baylor University Medical Center Proceedings, 2019
Tyler Smith, Mitchell Wong, Tove M. Goldson, Samuel N. Forjuoh
In the setting of WM, hyperviscosity syndrome is likely due to the accumulation of large IgM antibodies in smaller capillaries causing vascular congestion, which produces the more frequent symptoms of visual disturbances, dizziness, and headaches. One small study found that no patient with plasma viscosity <3 had symptoms of hyperviscosity syndrome and that symptoms usually begin to develop when serum viscosity is >4.8 Our patient was asymptomatic despite an initial serum viscosity of 4.2. With a level >4, it was reasonable to screen for retinal hemorrhages, which prompted the ophthalmology consult and the need for the funduscopic examination. Garcia-Sanz et al reported that approximately 34% of patients with WM had funduscopic abnormalities.7 Though these findings likely included overt hyperviscous patients, further questions arise regarding the development of retinal pathology along the spectrum of developing hyperviscosity syndrome. This ought to prompt the PCP to have a low threshold for ophthalmology referral even in asymptomatic patients and those without definite hyperviscosity syndrome. The 2016 Annual International Workshop on WM recommended that patients with serum IgM levels >3000 mg/dL be evaluated with funduscopic examination.9 For our patient, an ophthalmology referral was indicated based on a serum IgM of 6162.